A case is reported of a 60-year-old woman with acantholytic vesiculopustular dermatosis and IgA-λ monoclonal gammopathy. The histopathology of vesiculopustular lesions showed intraepidermal acantholytic and neutrophilic blisters. Direct immunofluorescence revealed intercellular (IC) IgG deposition with concurrent deposits of IgA and C3. Indirect immunofluorescence and immunoblotting studies revealed that the patient had circulating IgG and IgA anti-IC antibodies both of which recognized the 150-kD desmoglein that was pemphigus foliaceus antigen in a bovine desmosome preparation